Cognitive Deficits in Machado-Joseph Disease Correlate with Hypoperfusion of Visual System Areas

Cognitive Deficits in Machado-Joseph Disease Correlate with Hypoperfusion of Visual System Areas

Author Braga-Neto, Pedro Autor UNIFESP Google Scholar
Dutra, Livia Almeida Autor UNIFESP Google Scholar
Pedroso, Jose Luiz Autor UNIFESP Google Scholar
Felicio, Andre C. Autor UNIFESP Google Scholar
Alessi, Helena Autor UNIFESP Google Scholar
Santos-Galduroz, Ruth F. Google Scholar
Bertolucci, Paulo Henrique F. Autor UNIFESP Google Scholar
Castiglioni, Mario Luiz V. Autor UNIFESP Google Scholar
Bressan, Rodrigo Affonseca Autor UNIFESP Google Scholar
Jara de Garrido, Griselda Esther Google Scholar
Barsottini, Orlando Graziani Povoas Autor UNIFESP Google Scholar
Jackowski, Andrea Autor UNIFESP Google Scholar
Institution Universidade Federal de São Paulo (UNIFESP)
Universidade Federal do ABC (UFABC)
Univ Estadual Paulista
Univ Western Australia
Abstract Cognitive and olfactory impairments have previously been demonstrated in patients with spinocerebellar ataxia type 3 (SCA3), also known as Machado-Joseph disease (MJD)-SCA3/MJD. We investigated changes in regional cerebral blood flow (rCBF) using single-photon emission computed tomography (SPECT) imaging in a cohort of Brazilian patients with SCA3/MJD. the aim of the present study was to evaluate the correlation among rCBF, cognitive deficits, and olfactory dysfunction in SCA3/MJD. Twenty-nine genetically confirmed SCA3/MJD patients and 25 control subjects were enrolled in the study. the severity of cerebellar symptoms was measured using the International Cooperative Ataxia Rating Scale and the Scale for the Assessment and Rating of Ataxia. Psychiatric symptoms were evaluated by the Hamilton Anxiety Scale and Beck Depression Inventory. the neuropsychological assessment consisted of Spatial Span, Symbol Search, Picture Completion, the Stroop Color Word Test, Trail Making Test (TMT), and Phonemic Verbal Fluency. Subjects were also submitted to odor identification evaluation using the 16-item Sniffin' Sticks. SPECT was performed using ethyl cysteine dimer labeled with technetium-99m. SCA3/MJD patients showed reduced brain perfusion in the cerebellum, temporal, limbic, and occipital lobes compared to control subjects (pFDR < 0.001). A significant positive correlation was found between the Picture Completion test and perfusion of the left parahippocampal gyrus and basal ganglia in the patient group as well as a negative correlation between the TMT part A and bilateral thalamus perfusion. the visuospatial system is affected in patients with SCA3/MJD and may be responsible for the cognitive deficits seen in this disease.
Keywords Machado-Joseph disease
Spinocerebellar ataxia type 3
Cognitive deficits
Language English
Sponsor Fundação de Amparo à Pesquisa do Estado de São Paulo (FAPESP)
Conselho Nacional de Desenvolvimento Científico e Tecnológico (CNPq)
Instituto Albert Einstein de Ensino e Pesquisa
Date 2012-12-01
Published in Cerebellum. New York: Springer, v. 11, n. 4, p. 1037-1044, 2012.
ISSN 1473-4222 (Sherpa/Romeo, impact factor)
Publisher Springer
Extent 1037-1044
Access rights Closed access
Type Article
Web of Science ID WOS:000311249400023

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